Information on Bovine Spongiform Encephalopathy (BSE) in Canada
Informational Resources on BSE in Canada
Bovine Spongiform Encephalopathy (BSE)
A.K.A. Mad Cow Disease
What is Creutzfeldt-Jakob Disease?
(Creutzfeldt-Jakob is a human form of Spongiform Encephalopathy.)
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide; in the United States there are about 200 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of patients die within 1 year. In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.
There are three major categories of CJD:
In sporadic CJD, the disease appears even though the person has no known risk factors for the disease. This is by far the most common type of CJD and accounts for at least 85 percent of cases.
In hereditary CJD, the person has a family history of the disease and/or tests positive for a genetic mutation associated with CJD. About 5 to 10 percent of cases of CJD in the United States are hereditary.
In acquired CJD, the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. There is no evidence that CJD is contagious through casual contact with a CJD patient. Since CJD was first described in 1920, fewer than 1 percent of cases have been acquired CJD.
CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. CJD is the most common of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS). Kuru was identified in people of an isolated tribe in Papua New Guinea and has now almost disappeared. Fatal familial insomnia and GSS are extremely rare hereditary diseases, found in just a few families around the world. Other TSEs are found in specific kinds of animals. These include bovine spongiform encephalopathy (BSE), which is found in cows and often referred to as "mad cow" disease, scrapie, which affects sheep and goats, mink encephalopathy, and feline encephalopathy. Similar diseases have occurred in elk, deer, and exotic zoo animals. Courtesy of the National Institute of Neurological Diseases and Stroke (NINDS).
Backgrounder:
Bovine Spongiform Encephalopathy (BSE) - Canada and the US
Background information, and current situation reports, on the cases of BSE in Canada and the US.
BSE: Questions and Answers
Information from the Canadian Food Inspection Agency on the case of BSE in Canada.
General Information on BSE (Bovine Spongiform Encephalopathy)
The FDA's BSE / Mad Cow Disease Website
Information from the Food and Drug Administration on BSE / Mad Cow Disease, including information on the recent case in Canada.
BSE and CJD Information and Resources
Information from the Centers for Disease Control and Prevention (CDC) on Bovine Spongiform Encephalopathy (BSE) and its human counterpart, Creutzfeldt-Jakob disease (CJD).
DEFRA's BSE Home Page
Information from the Department of Environment, Food and Rural Affairs on BSE in the United Kingdom.
Official Mad Cow Disease Home Page
A consumer oriented website with a plethora of articles on Mad Cow Disease and its varient forms.
The information provided on this site is for informational purposes only.
Always consult your doctor for medical advice.
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